HLH, Hemophagocytic lymphohistiocytosis

HLH,噬血细胞淋巴组织细胞增生症
  • 文章类型: Case Reports
    未经证实:噬血细胞性淋巴组织细胞增生症(HLH)是一种与细胞因子风暴相关的侵袭性且危及生命的综合征。这里,我们介绍了1例合并结核分枝杆菌感染的获得性HLH患者.
    未经评估:我们报告了一名66岁的高血压和糖尿病男性患者,他有4天的发热和腹痛病史。否认咳嗽和体重减轻的病史。实验室调查显示:铁蛋白升高,C反应蛋白,和甘油三酯。骨髓检查显示>50%的噬血细胞(红细胞和血小板被巨噬细胞摄取),结核分枝杆菌的抗酸杆菌阳性,也没有恶性肿瘤的证据.全血细胞计数显示贫血和血小板减少。患者符合获得性噬血细胞性淋巴组织细胞增生症(HLH)的八项临床标准中的六项。患者接受了带有辅助类固醇的抗结核药物治疗。在随后的日子里,患者的临床症状明显改善,出院回家。然而,病人出院一周后去世。
    UASSIGNED:本案例强调了早期诊断和治疗与结核感染相关的获得性HLH的重要性,以改善患者的临床预后。
    UNASSIGNED: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome associated with cytokine storm. Here, we present a patient with acquired HLH associated with Mycobacterial tuberculosis infection.
    UNASSIGNED: We report a 66-year-old hypertensive and diabetic male patient who presented with four days history of fever and abdominal pain. Denied history of cough and weight loss. Laboratory investigation showed: elevated ferritin, C-reactive protein, and triglyceride. Bone marrow examination showed > 50% hemophagocytosis (RBCs and platelets ingested by macrophages), positive acid-fast bacillus for Mycobacterium tuberculosis bacilli, and no evidence of malignancy. Complete blood count showed anemia and thrombocytopenia. The patient fulfilled six out of eight clinical criterions of the acquired Hemophagocytic lymphohistiocytosis (HLH). The patient was managed with anti-tuberculous medications with adjuvant steroid. On the subsequent days, the patient showed significant clinical improvement and discharged home. However, the patient passed away a week after home discharge.
    UNASSIGNED: The present case highlights on the importance of early diagnosis and treatment of acquired HLH associated with tuberculous infection to improve the clinical outcome of the patient.
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  • 文章类型: Case Reports
    内脏利什曼病(VL)和巨噬细胞活化综合征(MAS)的组合由于其相似的临床表现而使诊断困难,预后不良,特别是因为治疗方法仍然缺乏编码。我们报告了一名来自Berkane的17个月大女性患者的病例,有3个月的无政府性发热病史,伴有由脸色苍白组成的贫血综合征和由鼻出血组成的出血性综合征。体格检查显示温度为39°C,下四肢水肿,皮肤和粘膜苍白,牙龈瘀斑,漂白的头发,和肝脾肿大.
    全血细胞计数显示全血细胞减少伴深度再生型正常细胞性贫血,浓度为3g/dL,白细胞4860/mm3,中性粒细胞减少680/mm3,血小板减少12.000/mm3,血涂片未见异常。这些异常与低白蛋白血症有关,高甘油三酯血症,高铁蛋白血症,乳酸脱氢酶(LDH)水平为337IU/L,低凝血酶原时间(PT)为56%,纤维蛋白原水平为1g/L。直接Coombs试验为阳性。脊髓造影检查显示存在利什曼原虫体和噬血细胞图。诊断为与MAS相关的内脏利什曼病。患者接受脂质体两性霉素B和皮质类固醇治疗,临床和生物进化良好,治疗耐受性良好。
    VL和MAS的关联仍然很少,即使在非地方病区也应引起,因为晚期诊断会使预后恶化,甚至可能导致患者的死亡,尽管进行了积极的治疗。
    UNASSIGNED: The combination of visceral leishmaniasis (VL) and macrophage activation syndrome (MAS) makes the diagnosis difficult due to their similar clinical presentation, with a poor prognosis especially since the treatment is still poorly codified.We report the case of a 17-month-old female patient from Berkane, presenting for a 3 months history of anarchic fever with anemic syndrome made up of pallor and hemorrhagic syndrome made up of epistaxis. Physical examination revealed a temperature of 39 ° C, lower limbsedema, paleness of skin and mucous membranes, gingival petechiae, bleached hair, and hepatosplenomegaly.
    UNASSIGNED: The complete blood count showed pancytopenia with deep aregenerative normochromic normocytic anemia at 3 g/dL, leukocytes were at 4860/mm 3 with neutropenia at 680/mm 3 and thrombocytopenia at 12.000/mm3, the blood smear was without abnormality. These anomalies were associated with a hypoalbunemia, hypertriglyceridemia, hyperferritinemia, lactate dehydrogenase (LDH) level was at 337 IU/L, low prothrombin time (PT) at 56 % and fibrinogen level at 1 g/L. The direct Coombs test was positive. Examination of the myelogram revealed the presence of leishmania bodies and figures of hemophagocytosis. A diagnosis of visceral leishmaniasis associated with MAS was made.The patient was put on liposomal amphotericin B and corticosteroid therapy with good clinical and biological evolution and good therapeutic tolerance.
    UNASSIGNED: The association of VL and MAS remains rare and should be evoked even in non-endemic areas since late diagnosis worsens the prognosis and may even be responsible for the death of patients despite an aggressive treatment.
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  • 文章类型: Journal Article
    噬血细胞淋巴组织细胞增生症是一种威胁生命的疾病,其特征是细胞毒性T淋巴细胞的持续病理激活,自然杀伤细胞,和巨噬细胞。我们介绍了一名高烧的年轻患者的详细信息,黄疸,和呼吸困难。关于调查,他得了肝炎,贫血,中性粒细胞减少症,和凝血病。他也有高甘油三酯血症,低纤维蛋白原血症,和高铁蛋白血症.骨髓穿刺显示组织细胞增生症,经颈静脉肝活检显示坏死性肉芽肿在抗酸杆菌染色上为结核分枝杆菌阳性。他通过免疫抑制剂和抗结核治疗的组合成功治疗。结核相关性噬血细胞综合征是罕见的,应考虑在患者的原因不明的噬血细胞综合征,尤其是在结核病流行地区。及时识别和抗结核治疗和免疫抑制剂治疗与良好的预后相关。
    Hemophagocytic lymphohistiocytosis is a life-threatening disorder characterized by persistent pathologic activation of cytotoxic T lymphocytes, natural killer cells, and macrophages. We present details of a young patient who presented with high-grade fever, jaundice, and breathlessness. On investigations, he had hepatitis, anemia, neutropenia, and coagulopathy. He also had hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Bone marrow aspiration revealed histiocytosis, and transjugular liver biopsy revealed necrotizing granulomas positive for Mycobacterium tuberculosis on acid-fast bacilli staining. He was successfully managed with a combination of immunosuppressants and antitubercular therapy. Tuberculosis associated hemophagocytosis syndrome is rare and should be considered in patients with unexplained hemophagocytosis syndrome, especially in tuberculosis-endemic regions. Prompt recognition and treatment with antitubercular treatment and immunosuppressants are associated with good outcomes.
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